¹Nehal  el  Ghobashy

¹Kasralainy Medical School

Introduction: Polyarteritis nodosa is a rare systemic necrotizing vasculitis of small and medium sized arteries that leads to thrombus, infractions and aneurysms in various organs. PAN is most common in males between 40 and 60 year and rare in childhood. The central nervous system is much less commonly involved than peripheral nervous system. We present  a case  of  juvenile   PAN with  cerebral aneurysm  as initial presentation which  was  successfully treated  with  embolization .  High dose of  steroids  and  cyclophosphamide  was  given as   induction  therapy  with  improvement.

Method(s): 6yrs old male patient presented with fever, weight loss, and livedo reticularis, then he developed headache with projectile vomiting. CT brain showed: subarachnoid hemorrhage. CT angiography was done: small saccular aneurysm along course of RT anterior temporal branch of RT middle cerebral artery. Two small mm aneurysm along distal course of RT anterior cerebral artery and occiptopariteal   branch of RT posterior cerebral artery .RT anterior temporal and RT superior cerebellar artery ruptured aneurysm for urgent embolization. The patient was referred to our department. Infectious causes for cerebral aneurysm were excluded. Pan cultures: negative. Echocardiography: normal. Serum titre for antinuclear antibody, ds-DNA, P-ANCA, C-ANCA were all-negative. The patient was diagnosed as PAN based on diagnostic criteria for childhood PAN (1).

High dose of steroids and cyclophosphamide were given with improvement.  The patient was maintained on low   dose of steroids and 100 mg of Azathioprine for 6 years.

2021: the patient stopped his treatment after which he presented with colicky abdominal pain, severe progressive vomiting, and bloody diarrhea. CT abdomen showed   small bowel obstruction with necrotizing enter colitis. Picture suggestive of mesenteric vascular occlusion .Resection and anastomosis was done. CT angiography of abdominal aorta & mesenteric vessels: patent abdominal aorta, its main branches (celiac, superior & inferior mesenteric as well as renal arteries. Patent portal vein   and its branches. No evidence of aneurysm or dissection.  The patient was   on steroids (0.5 mg/kg)   and mycophenolate mofetyl   (2gram).

2023: the patient started to complain of acute diminution of vision.  Urgent MRI, MRV and MRA were done . MRI brain: RT early  occipital subacute hematoma. RT cerebellar areas of   encephalomalacia.  MRV and MRA : notable tuft of  serpiginous arteries arising from the right middle cerebral artery .Mildly dilated cortical veins is noted at  the  overlying fossa reaching to  the superior sagittal  sinus . Attenuated left vertebral artery with dominant right one with normal MRV of  the  brain. Pulse  steroid was given  for  3days then 0.5 mg/ kg  of   oral  steroids  .Re  induction with  cyclophosamide was  started.Result(s): The patient  was  treated by  embolization, steroids  and cyclophosphamide

Conclusion(s): CNS involvement in PAN is rare aggressive treatment with steroids and cyclophosphamide is mandatory